A 43-year-old man with no significant medical history presents to his primary care provider (PCP) complaining of a "wound that won’t get better" on the left side of his neck. He states that the wound has been slowly growing over the past 2 years after it first appeared as a small pimple. In his efforts to heal the wound, he has used a variety of over-the-counter topical remedies such as hydrogen peroxide and triple antibiotic ointment; however, the wound has continued to spread and worsen. He was finally encouraged to visit his PCP when his brother noticed the now several-centimeters-long lesion (see Images 1-2). The patient denies having weight loss, fevers, or chills. He has not traveled during the past 5 years.
{mosimage}{mosimage}On physical examination, the patient is somewhat overweight. His vital signs are normal except for a blood pressure of 165/93 mm Hg. The examination of the head, eyes, ears, and nose is unremarkable. The patient has a 10-cm ulcer at the collar line on the left side of his neck. A homemade dressing that the patient had placed on this lesion contains a small amount of serosanguineous fluid. No lymphadenopathy and no masses are noted around the neck or in the armpits. The patient’s lungs are clear, and his heart rate is regular with normal heart sounds. The rest of the physical examination findings are unremarkable, except for numerous small hyperpigmented macules on the patient’s chest and back.
What is the most likely diagnosis, and what is the diagnostic test of choice?
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The ulcer is chronic, is nonhealing, and has a smooth border.
Authors:
Anusuya Mokashi,
Medical Student,
New York Medical College,
Valhalla, NY
Senait Dyson, MD,
Director,
Dermatology-Dermatopathology Lab,
UC Irvine, Irvine, Calif
eMedicine Editors:
Brady Pregerson, MD,
Depts. of Emergency Medicine Cedars-Sinai Medical Center Los Angeles, Calif,
Tri-City Medical Center Oceanside, Calif
Rick G. Kulkarni, MD,
Assistant Professor,
Yale School of Medicine,
Section of Emergency Medicine,
Department of Surgery,
Attending Physician,
Medical Director,
Department of Emergency Services,
Yale-New Haven Hospital, Conn
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ANSWER
Basal cell carcinoma (BCC): The diagnosis is advanced BCC of the neck. Most skin cancers can be placed into 1 of 3 categories: malignant melanomas, which have the highest rate of metastasis; BCCs, which almost never metastasize but can be locally invasive; and squamous cell carcinomas (SCCs), which are locally invasive and also occasionally metastasize.
BCC is the most common skin cancer and malignancy in the United States. The estimated lifetime risk of BCC in the Caucasian population is 33-39% in men and 23-28% in women, with a male-to-female ratio of 3:2. Fair-skinned people (as determined by using tools such as the Fitzpatrick skin scale) have an increased susceptibility to sun damage and BCC. Fair-skinned individuals of Celtic ancestry have the highest risk of BCC; the incidence of BCC is relatively low in people of African, Asian, or Hispanic descent. The incidence of BCC peaks at 55-75 years of age. Mortality is extremely rare with BCC; however, cosmetic disfigurement from both the tumor and the surgical therapy is an important consideration.
Exposure to UV radiation is the most common and important etiologic factor for BCC. The UV spectrum of sunlight is divided into long-wave (UV-A, 320-400 nm), intermediate-wave (UV-B, 290-320 nm), and short-wave (UV-C, 200-290 nm). UV-B and UV-C can alter nucleic acid bonds, leading to mutations caused by the activation of oncogenes or by the inactivation of tumor suppressor genes. UV-C does not penetrate the atmospheric ozone layer; therefore, UV-B is the primary culprit for most skin cancers. Areas of skin exposed to the sun, such as the head and neck, are affected in 75-80% of cases. The nose, specifically the nasal tip and alae, are common locations for BCC.
Given the relationship between BCC and UV light, the US incidence of skin cancer is highest in Texas and Arizona, where about 300 out of 100,000 people are affected. In contrast, the incidence is about half that number in Maine and New York. Worldwide, the incidence is highest in Australia, where 650-1560 out of 100,000 individuals develops skin cancer.
Other etiologic and risk factors associated with BCC are exposure to other forms of radiation, arsenic exposure, immunosuppression, xeroderma pigmentosa, Bazex syndrome, and previous nonmelanoma skin cancers.
Seven clinical and histologic subtypes of BCC are described. The most common form is nodular BCC, which typically results in waxy papules with a central depression, a pearly appearance, erosions or ulcerations, crusting, rolled or raised borders, translucency, telangiectasias over the surface, and a history of bleeding with minor trauma. Pigmented BCCs contain increased amounts of brown or black pigment and are most common in individuals with dark skin. Cystic BCCs are translucent blue-gray cystic nodules that may mimic benign cystic lesions. Superficial BCCs appear as scaly patches or papules that are pink to red-brown, often with central clearing. A threadlike border is common with superficial BCCs, and lesions usually occur on the trunk. The papules may mimic psoriasis or eczema, but they are slowly progressive and are not prone to fluctuations in appearance. Micronodular BCCs may appear yellow-white when stretched, and they are firm to the touch. They may have seemingly well-defined borders and may metastasize. Morpheaform and infiltrating BCCs can also metastasize and cause sclerotic plaques or papules. The borders are usually ill defined and often extend well beyond clinical margins. Ulceration, bleeding, and crusting are not common with morpheaform BCCs, which may be mistaken for scar tissue.
Skin cancers, such as BCCs, are best diagnosed by means of a shave or punch biopsy of the lesion. On histologic evaluation, a basaloid appearance of epithelial islands is a pathognomonic feature. The cells of BCC mimic germinative epithelium and have an increased nucleus-to-cytoplasm ratio.
Once the diagnosis is confirmed, BCCs can be treated medically or with surgical excision. Local therapy with chemotherapeutic and immunomodulating agents, such as 5-fluorouracil, interferon alfa-2b, and imiquimod, may be used to manage superficial and small lesions. In individuals with a history of BCC, 5-fluorouracil is used to treat subclinical lesions on areas of chronic sun exposure or on areas at risk (eg, basal cell nevus syndrome). Surgical options for treating BCC include curettage, excision, and cryotherapy. On areas of cosmetic concern, such as the face, Mohs micrographic techniques are used to minimize damage to healthy tissue and to improve the cosmetic outcome. When tumors are advanced, radiation therapy can be considered in addition to surgery. The patient in this case was treated by surgical excision.
Measures to prevent BCC include wearing protective clothing and sunscreens and avoiding sun exposure during peak hours of UV radiation, which are between 10 am to 4 pm.
References:
Ramsey, ML. Basal Cell Carcinoma. eMedicine from WebMD. Updated May 9, 2006. Available at: http://www.emedicine.com/derm/topic47.htm. Accessed 4/19/2007.
Nickoloff BJ, Qin JZ, Chaturvedi V, Bacon P, Panella J, Denning MF. Life and Death Signaling Pathways Contributing to Skin Cancer. J Invest Dermatol. 2002 Dec;7(1):27-35. [MEDLINE: 17529955]
Lackey PL, Sargent LA, Wong L, Brzezienski M, Kennedy JW. Giant basal cell carcinoma surgical management and reconstructive challenges. Ann Plast Surg. 2007 Mar;58(3):250-4. [MEDLINE: 17471127]
Massari LP, Kastelan M, Gruber F. Epidermal malignant tumors: pathogenesis, influence of UV light and apoptosis. Coll Antropol. 2007 Jan;31 Suppl 1:83-5. [MEDLINE: 17469758]
Kuijpers DI, Thissen MR, Berretty PJ, Ideler FH, Nelemans PJ, Neumann MH. Surgical Excision versus Curettage plus Cryosurgery in the Treatment of Basal Cell Carcinoma. Dermatol Surg. 2007 May;33(5):579-87. [MEDLINE: 17451581]
